Evaluating N-Acetylcysteine in CTD-ILD Treatment

Specific Aim 1:

The first objective of this protocol is to assess the tolerability and safety of N-acetylcysteine (NAC) in patients with connective tissue disease related interstitial lung disease (CTD-ILD).

The investigators will assess tolerability and safety at scheduled intervals via standard of care physical exam findings, monitoring complete blood count, serum chemistry, and pulmonary function tests, along with patient reports of medication intolerance and change in symptoms of dyspnea.

Specific Aim 2:

The second objective of this protocol is to evaluate the efficacy of NAC in patients with CTD-ILD.

The investigators will evaluate efficacious treatment by assessing pulmonary function tests and subjective symptoms reported on patient questionnaires before starting NAC and again after three, six, nine, and twelve months of treatment initiation.

Specific Aim 3:

The third objective of this protocol is to assess serum matrix metalloproteinase (MMP) levels as a potential biomarker.

Previous studies have documented elevated MMP levels in fibrotic lung disease. This prospective cohort study in patients with CTD-ILD will evaluate whether serum MMP is associated with clinically meaningful changes, and evaluate if levels correlate with changes in physiology or biomarkers.